A12-day-old asymptomatic neonate was detected to have multiple cardiac masses during evaluation of a cardiac murmur. There were multiple lobulated cardiac masses in the left ventricular apical region and interventricular septum (largest 10 x 8 mm) and pedunculated mass (14 × 9 mm) in right ventricular outflow tract (RVOT) (Fig. 1). The baby did not have other features of tuberous sclerosis and was kept on close medical follow-up.

Fig. 1 Echocardiogr am (parasternal long-axis view) showing multiple cardiac masss in left ventricle (LV) and right ventricle (RV).

At 7 months of life, parents reported failure to thrive and new onset feeding difficulty. The right ventricular mass had increased in size (19×19 mm) and was causing severe RVOT obstruction (peak gradient 86 mmHg) without increase in size of left ventricular masses. In view of symptomatic severe RVOT obstruction, surgical resection of all the masses was done. The largest mass (20×15 mm) was firm in consistency, gray-white and glistening, arising from right ventricular free wall partly attached to the chordae of septal leaflet of tricuspid valve (Web Fig. 1a). Histopathology showed vacuolated tumor cells with clear cytoplasm and characteristic spider cells on Haematoxylin and Eosin staining (Web Fig. 1b) and Desmin expression (Web Fig. 1c) suggestive of cardiac rhabdomyoma.

Neonatal cardiac tumours are rare, rhabdomyomas being commonest among them. Tuberous sclerosis is associated with cardiac rhabdomyomas in 50-60% patients and conversely, rhabomyomas are associated with tuberous sclerosis in 59-80% [1,2]. Rhabdomyomas are generally multiple, well-circumscribed, intramural or pedunculated tumours seen most commonly in the ventricles. They are hamartomas with no malignant potential. Their presentation varies from asymptomatic incidentally detected cardiac murmur, congestive cardiac failure, arrhythmias or sudden infant death depending on the size, number and location of the tumour.

Cardiac rhabdomyomas have a propensity for spontaneous regression [3,4]. Most of them have a benign course and remain static or regress with age, higher chances of spontaneous regression seen at younger age. Complete regression is common in the ûrst 4 years of life [3,4]. Mammalian targets for rapamycin inhibitors have been used to treat large, inoperable or residual rhabdomyomas [5]. Surgical intervention is indicated with haemodynamic compromise or intractable arrhythmia. Progressive severe dynamic outflow tract obstruction is an uncommon presentation and surgery can be life-saving.